Definition, Etiology, Clinical FeaturesTop
Erythromelalgia is a vasomotor disorder associated with acute (and sometimes followed by chronic) episodic dilation of the arterioles and arteriovenous connections. It manifests as sudden erythema and warmth of the extremities, particularly fingers and toes, that involves the feet more often than the hands and is accompanied by severe burning pain. The features are usually symmetric and sometimes also occur on the ears and face. Each episode can last for hours to years.
Causes: Secondary erythromelalgia develops in the course of myeloproliferative neoplasms (particularly essential thrombocythemia and polycythemia vera), systemic connective tissue diseases, type 1 or type 2 diabetes mellitus, multiple sclerosis, neuropathy of various etiologies, infectious diseases (eg, AIDS), and peripheral embolism (especially cholesterol emboli). It may also be caused by certain drugs (bromocriptine, nifedipine) or trauma.
Symptoms of erythromelalgia may be improved by cooling and elevating the affected extremity and by avoiding high temperatures and strenuous exercise. In some patients sedatives and pharmacologic nerve blockade may be helpful. Treatment, particularly in the case of secondary erythromelalgia, involves acetylsalicylic acid (ASA) up to 100 mg/d, indomethacin, propranolol, serotonin reuptake inhibitors (eg, sertraline), and clonazepam. In case of severe pain, you may consider IV infusion of sodium nitroprusside, lidocaine, or prostaglandin E1.